Background and purpose
In this retrospective study involving 14 university hospitals from France and Switzerland, the aim was to define the clinicopathological features of chronic neuropathies with ...anti‐disialosyl ganglioside immunoglobulin M (IgM) antibodies (CNDA).
Results
Fifty‐five patients with a polyneuropathy evolving for more than 2 months and with at least one anti‐disialosyl ganglioside IgM antibody, that is, anti‐GD1b, ‐GT1b, ‐GQ1b, ‐GT1a, ‐GD2 and ‐GD3, were identified. Seventy‐eight percent of patients were male, mean age at disease onset was 55 years (30–76) and disease onset was progressive (82%) or acute (18%). Patients presented with limb sensory symptoms (94% of cases), sensory ataxia (85%), oculomotor weakness (36%), limb motor symptoms (31%) and bulbar muscle weakness (18%). Sixty‐five percent of patients had a demyelinating polyradiculoneuropathy electrodiagnostic profile and 24% a sensory neuronopathy profile. Anti‐GD1b antibodies were found in 78% of cases, whilst other anti‐disialosyl antibodies were each observed in less than 51% of patients. Other features included nerve biopsy demyelination (100% of cases), increased cerebrospinal fluid protein content (75%), IgM paraprotein (50%) and malignant hemopathy (8%). Eighty‐six percent of CNDA patients were intravenous immunoglobulins‐responsive, and rituximab was successfully used as second‐line treatment in 50% of cases. Fifteen percent of patients had mild symptoms and were not treated. CNDA course was progressive (55%) or relapsing (45%), and 93% of patients still walked after a mean disease duration of 11 years.
Conclusion
Chronic neuropathies with anti‐disialosyl ganglioside IgM antibodies have a recognizable phenotype, are mostly intravenous immunoglobulins‐responsive and present with a good outcome in a majority of cases.
Les syndromes neurologiques paranéoplasiques (SNP) sont des complications rares des cancers survenant dans 0,01 % des cas. Leur présentation clinique, biologique et radiologique est hétérogène et ...peut constituer un challenge diagnostique.
Un patient de 29 ans sans antécédent particulier est adressé aux urgences pour syndrome confusionnel avec céphalées et troubles de l’équilibre évoluant depuis 5 mois. L’examen clinique initial montre un ralentissement psychomoteur marqué, avec une désorientation temporospatiale, une hypoacousie bilatérale, une dysarthrie et un syndrome cérébelleux statique. L’IRM cérébrale révèle un processus expansif intracrânien de la région commissurale antérieure étendue aux régions hypothalamique et amygdalienne gauches avec prise de contraste homogène. La ponction lombaire montre une méningite lymphocytaire sans cellule tumorale. Une biopsie cérébrale est réalisée retrouvant un infiltrat inflammatoire sans cellules tumorales. Un bilan systémique est réalisé retrouvant une lésion du médiastin. Une exérèse chirurgicale de la lésion médiastinale est réalisée. L’examen histologique conclut à tumeur germinale séminomateuse. Une chimiothérapie systémique par cisplatine et étoposide est débutée. Malgré une bonne réponse initiale à la corticothérapie, une paralysie oculomotrice verticale apparaît et le syndrome confusionnel se majore. L’IRM cérébrale montre alors une quasi-régression des prises de contraste initiales. La discordance évolutive clinicoradiologique conduit à la recherche d’anticorps anti-neuronaux qui revient positive aux anticorps anti-Ma2.
Un traitement par Immunoglobulines polyvalente, rituximab et cyclophosphamide est réalisé avec une évolution neurologique lentement favorable. Les SNP à anti-Ma2 sont des maladies rares au pronostic réservé. Ils sont le plus souvent associés à une tumeur testiculaire type séminomateuse. Classiquement, ils se présentent par une encéphalite limbique, diencéphalique et/ou du tronc cérébral.
Les lésions tumorales dans ces localisations doivent faire évoquer un SNP pour instaurer un traitement rapide et adapté.
Abstract
Context
Recombinant human thyrotropin (rhTSH) has been shown to be an effective stimulation method for radioactive iodine (RAI) therapy in differentiated thyroid cancer, including in those ...with nodal metastases (N1 DTC).
Objectives
To demonstrate the noninferiority of rhTSH vs thyroid hormone withdrawal (THW) in preparation to RAI regarding disease status at the first evaluation in the real-life setting in patients with N1 DTC.
Design
This was a French multicenter retrospective study. Groups were matched according to age (<45/≥45 years), number of N1 nodes (≤5/>5 lymph nodes), and stage (pT1-T2/pT3).
Results
The cohort consisted of 404 patients pT1-T3/N1/M0 DTC treated with rhTSH (n = 205) or THW (n = 199). Pathological characteristics and initially administrated RAI activities (3.27 ± 1.00 GBq) were similar between the two groups. At first evaluation (6 to 18 months post-RAI), disease-free status was defined by thyroglobulin levels below threshold and a normal ultrasound. Disease-free rate was not inferior in the rhTSH group (75.1%) compared with the THW group (71.9%). The observed difference between the success rates was 3.3% (−6.6 to 13.0); rhTSH was therefore considered noninferior to THW because the upper limit of this interval was <15%. At the last evaluation (29.7 ± 20.7 months for rhTSH; 36.7 ± 23.8 months for THW), 83.5% (rhTSH) and 81.5% (THW) of patients achieved a complete response. This result was not influenced by any of the known prognostic factors.
Conclusions
A preparation for initial RAI treatment with rhTSH was noninferior to that with THW in our series of pT1-T3/N1/M0-DTC on disease-free status outcomes at the first evaluation and after 3 years.
In a French retrospective multicenter matched study, rhTSH has been shown non-inferior to THW for RAI therapy in DTC patients staged pT1-T3/N1/M0, independently from the evaluated prognostic factors.
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